The sudden cardiac death (SCD) of a young person is a devastating event, with the incidence estimated at between one to seven deaths per 100,000 people per year in in…
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Sudden unexpected cardiac death in young people (aged 1–40) occurs at a rate of over 100 people per year in New Zealand. To date, we know that about one-third have a familial cause, and death can be prevented in these people, but they must first be detected in the community. This study shows that by screening family members of people who carry such a condition, with blood tests and heart tests, it is possible to detect large numbers of people at risk and offer them protection. It also shows that this is being done much better in the North Island than the South Island, because the South Island does not have anyone to coordinate the service and keep records. The authors argue that there is a pressing need for a South Island coordinator to address this inequity of service.
To investigate regional variations in the detection of sudden death syndromes across New Zealand by assessing registrations in the national Cardiac Inherited Diseases Registry New Zealand (CIDRNZ).
The CIDRNZ has been a national entity since 2009, with a hub in Auckland and locally funded regional coordinators (Midland, Central) linked with multidisciplinary cardiac genetic teams. Registration is consent-based and voluntary, and involves the collection of clinical/genetic information and permits genetic testing and research. Registry data were extracted from the CIDRNZ in October 2015 and results are expressed as registrations per 100,000 people by district health board area.
The CIDRNZ has 1,940 registrants from 712 families, 46% of whom are definitely or probably affected by cardiac inherited disease. There are clear regional differences in registration frequencies between regions and between the North and South Islands, both for overall registrations (56/100,000 and 14/100,000, respectively; p<0.001) and for long QT syndrome registrations (15/100,000 and 6/100,000, respectively; p<0.001). Regions with local coordinators have the highest number of registrations.
The detection of sudden death syndromes in New Zealand through a cardiac genetic registry is possible but much work is needed to improve regional variation in the detection/reporting of these conditions across the country.